New Study Highlights Complex Role of Immune System in ALS

Washington, D.C. (July 11, 2013)—In work supported by The ALS Association, researchers have identified the molecular signature of immune cells involved in the ALS disease process and used it to better understand the disease-related role of these cells, which surround dying motor neurons. The study was published in the journal Cell Reports.

ALS (amyotrophic lateral sclerosis) is a progressive neurodegenerative disease that affects neurons (nerve cells) in the brain and the spinal cord. Eventually, people with ALS lose the ability to initiate and control muscle movement, which often leads to total paralysis and death within two to five years of diagnosis. There is no cure and no life-prolonging treatments for the disease.

The researchers studied microglia, a type of immune cell found in the central nervous system (CNS). They identified 29 genes that distinguish microglia from other cells in the CNS. Using these as cell-specific markers, they were able to study the proliferation and movements of microglia during the ALS disease process in ALS mice in unprecedented detail. They found that the microglia may have both neurotoxic and neuroprotective effects. This result may suggest that treatments that broadly suppress the immune system may not be the most effective strategy against the disease, but further work will be needed to explore that possibility.

“This study provides an important new tool to study the role of these cells in ALS,” said Lucie Bruijn, Ph.D., Chief Scientist for The Association. “We can now use this tool to answer questions about the disease process, which we have not been able to ask before. That should help us determine the best way to manipulate the immune system to aid in fighting the disease.”

The research was performed by Isaac Chiu, Ph.D., of Harvard Medical School, and colleagues, under the supervision of Tom Maniatis, Ph.D., of Columbia University Medical Center with the assistance of Richard Myers, Ph.D., of the Hudson Alpha Institute for Biotechnology in Huntsville, Alabama. Dr. Maniatis’s work is supported by the Greater New York Chapter of The ALS Association, and Dr. Myers’s work is supported by the Alabama Chapter of The ALS Association.

About The ALS AssociationThe ALS Association is the only national non-profit organization fighting Lou Gehrig’s Disease on every front. By leading the way in global research, providing assistance for people with ALS through a nationwide network of chapters, coordinating multidisciplinary care through certified clinical care centers, and fostering government partnerships, The Association builds hope and enhances quality of life while aggressively searching for new treatments and a cure. For more information about The ALS Association, visit our website at www.alsa.org.